OBJECTIVE: To evaluate the baseline clinical characteristics of juvenile systemic sclerosis (SSc) patients in the international juvenile SSc inception cohort, and to compare these characteristics between the classically defined juvenile diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc) subtypes and among those with overlap features. METHODS: A cross-sectional study was performed using baseline visit data. Information on demographic characteristics, organ system evaluation, treatment, and patient- and physician-reported outcomes was extracted and summary statistics applied. Comparisons between juvenile dcSSc and lcSSc subtypes and patients with and without overlap features were performed using chi-square and Mann-Whitney U tests. RESULTS: At data extraction, 150 juvenile SSc patients were enrolled across 42 centers; 83% were White, 80% were female, juvenile dcSSc predominated (72%), and 17% of the cohort had overlap features. Significant differences were found between juvenile dcSSc and juvenile lcSSc regarding modified Rodnan skin thickness score, the presence of Gottron's papules, digital tip ulceration, results of the 6-minute walk test, and composite pulmonary and cardiac involvement. All of these were more frequent in dcSSc except for cardiac involvement. Juvenile dcSSc patients had significantly worse scores for physician-rated disease activity and damage. A significantly higher occurrence of Gottron's papules and musculoskeletal and composite pulmonary involvement, and a significantly lower frequency of Raynaud's phenomenon, were seen in those with overlap features. CONCLUSION: Results from a large international juvenile SSc cohort demonstrate significant differences between juvenile dcSSc and juvenile lcSSc patients, including more globally severe disease and increased frequency of interstitial lung disease in juvenile dcSSc patients, while those with lcSSc have more frequent cardiac involvement. Those with overlap features had an unexpected higher frequency of interstitial lung disease.

Asklepios Klnik Sankt Augustin Sankt Augustin Germany

Cerrahpasa Medical School Istanbul University Cerrahpasa Istanbul Turkey

Charité University Medicine and German Rheumatism Research Center Berlin Berlin Germany

Charles University Prague Czech Republic

Children's Hospital at Montefiore Bronx New York

Clinical Center Nis University of Niš Nis Serbia

Emma Children's Hospital Amsterdam University Medical Centers University of Amsterdam Amsterdam The Netherlands

German Rheumatism Research Center Berlin Germany

Ghent University and Ghent University Hospital Ghent Belgium

Great Ormond Street Hospital for Children NHS Foundation Trust London UK

Hamburg Centre for Pediatric and Adolescent Rheumatology Schön Klinik Hamburg Eilbek Hamburg Germany

Hospital de Niños Dr Orlando Alassia Santa Fe Argentina

Hospital de Pediatria J P Garrahan Buenos Aires Argentina

Hospital de Santa Maria Universidade de Lisboa Lisbon Portugal

Hospital for Special Surgery New York New York

Hospital Garcia de Orta Almada Portugal

Hospital Sant Joan de Déu Esplugues Universitat de Barcelona Barcelona Spain

Hospital Universitário Clementino Fraga Filho and Universidade Federal do Rio de Janeiro Rio de Janeiro Brazil

Luzerner Kantonsspital Kinderspital Luzern Switzerland

Medical University Innsbruck Innsbruck Austria

Meir Medical Center Kfar Saba Tel Aviv University Tel Aviv Israel

Meyer Children's Hospital Florence Italy

National Medical Research Center of Children's Health Sechenov 1st Moscow State Medical University of the Ministry of Health of the Russian Federation Moscow Russia

Riga Stradins University University Children Hospital Riga Latvia

Rigshospitalet Copenhagen Denmark

Saint Petersburg State Pediatric Medical University Saint Petersburg Russia

Schneider Children's Medical Center of Israel Sackler Faculty of Medicine Tel Aviv University Petah Tikva Israel

School of Medical Science University of Campinas Sao Paulo Brazil

SickKids The Hospital for Sick Children Toronto Ontario Canada

Sir Ganga Ram Hospital Delhi India

Sri Ramachandra University Chennai India

Stony Brook Children's Hospital Stony Brook New York

Universidade do Estado Rio de Janeiro Brazil

Universidade Federal de São Paulo Sao Paulo Brazil

University Children's Hospital University Medical Center Ljubljana Ljubljana Slovenia

University Hospital Ramón y Cajal Madrid Spain

University of Missouri Columbia

University of Pittsburgh Children's Hospital of Pittsburgh Pittsburgh Pennsylvania

University Tuebingen Tuebingen Germany

Uppsala University Uppsala Sweden

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$a Foeldvari, Ivan $u Hamburg Centre for Pediatric and Adolescent Rheumatology, Schön Klinik Hamburg Eilbek, Hamburg, Germany $1 https://orcid.org/0000000306595298

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$a Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort / $c I. Foeldvari, J. Klotsche, O. Kasapcopur, A. Adrovic, MT. Terreri, AP. Sakamoto, V. Stanevicha, F. Sztajnbok, J. Anton, B. Feldman, E. Alexeeva, M. Katsicas, V. Smith, T. Avcin, E. Marrani, M. Kostik, T. Lehman, WA. Sifuentes-Giraldo, N. Vasquez-Canizares, S. Appenzeller, M. Janarthanan, M. Moll, D. Nemcova, A. Patwardhan, MJ. Santos, S. Sawhney, D. Schonenberg-Meinema, C. Battagliotti, L. Berntson, B. Bica, J. Brunner, P. Costa-Reis, D. Eleftheriou, L. Harel, G. Horneff, D. Kaiser, T. Kallinich, D. Lazarevic, K. Minden, S. Nielsen, F. Nuruzzaman, Y. Uziel, N. Helmus, KS. Torok

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$a OBJECTIVE: To evaluate the baseline clinical characteristics of juvenile systemic sclerosis (SSc) patients in the international juvenile SSc inception cohort, and to compare these characteristics between the classically defined juvenile diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc) subtypes and among those with overlap features. METHODS: A cross-sectional study was performed using baseline visit data. Information on demographic characteristics, organ system evaluation, treatment, and patient- and physician-reported outcomes was extracted and summary statistics applied. Comparisons between juvenile dcSSc and lcSSc subtypes and patients with and without overlap features were performed using chi-square and Mann-Whitney U tests. RESULTS: At data extraction, 150 juvenile SSc patients were enrolled across 42 centers; 83% were White, 80% were female, juvenile dcSSc predominated (72%), and 17% of the cohort had overlap features. Significant differences were found between juvenile dcSSc and juvenile lcSSc regarding modified Rodnan skin thickness score, the presence of Gottron's papules, digital tip ulceration, results of the 6-minute walk test, and composite pulmonary and cardiac involvement. All of these were more frequent in dcSSc except for cardiac involvement. Juvenile dcSSc patients had significantly worse scores for physician-rated disease activity and damage. A significantly higher occurrence of Gottron's papules and musculoskeletal and composite pulmonary involvement, and a significantly lower frequency of Raynaud's phenomenon, were seen in those with overlap features. CONCLUSION: Results from a large international juvenile SSc cohort demonstrate significant differences between juvenile dcSSc and juvenile lcSSc patients, including more globally severe disease and increased frequency of interstitial lung disease in juvenile dcSSc patients, while those with lcSSc have more frequent cardiac involvement. Those with overlap features had an unexpected higher frequency of interstitial lung disease.

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$a Klotsche, Jens $u German Rheumatism Research Center, Berlin, Germany $1 https://orcid.org/0000000229545755

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$a Adrovic, Amra $u Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey

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$a Sakamoto, Ana Paula $u Universidade Federal de São Paulo, Sao Paulo, Brazil

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$a Avcin, Tadej $u University Children's Hospital University Medical Center Ljubljana, Ljubljana, Slovenia

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$a Marrani, Edoardo $u Meyer Children's Hospital, Florence, Italy $1 https://orcid.org/0000000203463268

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$a Kostik, Mikhail $u Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia

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$a Battagliotti, Cristina $u Hospital de Niños Dr. Orlando Alassia, Santa Fe, Argentina

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$a Berntson, Lillemor $u Uppsala University, Uppsala, Sweden

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$a Bica, Blanca $u Hospital Universitário Clementino Fraga Filho and Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil

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$a Brunner, Jürgen $u Medical University Innsbruck, Innsbruck, Austria

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$a Costa-Reis, Patricia $u Hospital de Santa Maria, Universidade de Lisboa, Lisbon, Portugal

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$a Harel, Liora $u Schneider Children's Medical Center of Israel Sackler Faculty of Medicine, Tel Aviv University, Petah-Tikva, Israel

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$a Kaiser, Daniela $u Luzerner Kantonsspital, Kinderspital, Luzern, Switzerland

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$a Lazarevic, Dragana $u Clinical Center Nis, University of Niš, Nis, Serbia

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$a Nielsen, Susan $u Rigshospitalet, Copenhagen, Denmark

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$a Nuruzzaman, Farzana $u Stony Brook Children's Hospital, Stony Brook, New York

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$a Uziel, Yosef $u Meir Medical Center, Kfar Saba, Tel Aviv University, Tel Aviv, Israel

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$a Helmus, Nicola $u Hamburg Centre for Pediatric and Adolescent Rheumatology, Schön Klinik Hamburg Eilbek, Hamburg, Germany

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